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Neurological Conditions and Diseases (During Development)


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  • 1. Neurological conditions and diseases Post Basic Paediatrics 18 April 2012
  • 2. Neurological conditions and diseasesPart I– At birth (Congenital, acquired) Macrocephaly Microcephaly Spine defect Other developmental defect Birth trauma/HIEPart II– During development (Congenital, acquired) Meningitis Seizure Headache Stroke/Vascular Neoplasm/Tumour Trauma Coma
  • 3. Neurological conditions and diseases Part II During development (Congenital, acquired)
  • 4. During development (Congenital, acquired)– Meningitis– Seizure– Headache– Stroke/Vascular– Neoplasm/Tumour– Trauma– Coma
  • 5. MeningitisInflammation of the meningesMost common infection of the CNSMost cases (70%) occur in children <5 years oldMay be secondary to other localized or systemic infections(e.g., otitis media).Two primary classifications– Viral– Bacterial– Others: fungi and (rarely) parasitesMorbidity is also high.About 30% of survivors have some sequelae of theirdiseaseHowever, these complications can be reduced if meningitisis treated early.
  • 6. AssessmentViral meningitis– Infants and toddlers Irritability, lethargy, vomiting Change in appetite– Older children Usually preceded by a nonspecific febrile illness Headache, malaise, muscle aches, nausea/ vomiting, photophobia, nuchal/spinal rigidity
  • 7. AssessmentBacterial meningitis– Infants and toddlers Poor feeding/suck, vomiting, high-pitched cry, bulging fontanel, fever or hypothermia, poor muscle tone– Children and adolescents Abrupt onset Fever, chills, headache, nuchal rigidity, vomiting, changes in LOC, photophobia, extreme irritability
  • 8. AssessmentIn children <1 month old:– group B Streptococcus, Escherichia coliIn children 4-12 weeks old:– E. coli, Hemophilus influenzae type B, Streptococcus pneumoniae, group B Streptococcus, Neisseria meningitidis (meningococcal)In children 3 months to 18 years old:– Streptococcus pneumoniae (most common cause), N. meningitidis, H. influenza type B (rare)– Mycobacterium tuberculosis
  • 9. AssessmentPHYSICAL EXAM- Temperature- elevated- Tachycardia or bradycardia with increased intracranial pressure- Blood pressure normal (low if septic shock has occurred)- Child in moderate-to-acute distress- Flushed- Level of consciousness variable- Possible enlargement of the cervical nodes- Focal neurologic signs: - photophobia - nuchal rigidity (in children >12 months old) - positive Brudzinskis sign ( in children >12 months) - positive Kernigs sign (in children >12 months )
  • 10. Differential DiagnosisBacteremiaSepsisSeptic shockBrain abscess
  • 11. ComplicationSeizuresComaBlindnessDeafnessDeathPalsies of cranial nerves III, VI, VII,VIII
  • 12. Lumbar puncturePatients at greatest risk for meningitis – under 18 months of age – seizure in the ED – focal or prolonged seizure – seen a physician within the past 48 hoursOther indications – concern about follow-up – prior treatment with antibioticsThe American Academy of Pediatrics “strongly consider” in infants under 12 months of age with a first febrile seizure
  • 13. Management: Nursing Interventions Place child in isolation until 24 hours of antibiotic therapy has completed Administer antibiotics (7-14 days) Fever control Monitor for signs of ICP Monitor for fluid overload Viral meningitis is treated symptomatically
  • 14. Prevention and ControlMeningitis Caused by Hemophilus influenzae– A vaccine is now routinely given to infants as part of the usual childhood immunizations.
  • 15. During development (Congenital, acquired)– Meningitis– Seizure– Headache– Stroke/Vascular– Neoplasm/Tumour– Trauma– Coma
  • 16. SeizureFebrile seizureEpilepsy - two or more seizures notprovoked by a specific event such as fever,trauma, infection, or chemical changeNeonatal seizuresStatus epilepticus
  • 17. Febrile seizure Definition: Convulsions occurring in association with fever in children between 3 months and 6 years of age, in whom there is no evidence of intracranial pathology or metabolic derangement.- No signs or history of underlying seizure disorder- Often familial- Uncomplicated and benign if seizure is of short duration (<15 minutes), only 1 in 24 hours, and normal CNS exam after seizure- Involves tonic-clonic movements, bilaterally- Associated with temperature >38°C
  • 18. EpidemiologyAge 3mo – 5yrsPeak age 9-20 months2-5% children will have before age 525-40% will have family history80 – 97% simple3 - 20% complex
  • 19. ManagementControl fever – take off clothing and tepid sponging. – antipyretic e.g. syrup or rectal Paracetamol 15 mg/kg 6 hourly. – antipyretic is indicated for parent’s comfort, but has not been shown to reduce the recurrence rate of febrile convulsion.
  • 20. EpilepsyDefinition: a neurological conditioncharacterised by recurrent unprovokedepileptic seizuresILAE Classification of seizure types:– generalized LOC tonic, clonic, tonic-clonic, myoclonic, atonic, absence– partial – focal onset simple partial – no LOC complex partial – LOC partial secondarily generalized– unclassified
  • 21. Epilepsy: Etiologyinfectiousmetabolictraumatictoxicneoplastic
  • 22. Epilepsy: Differential diagnosis
  • 23. Neonatal seizurebrief and subtle – eye blinking – mouth/tongue movements – “bicycling” motion to limbstypically seizure’s can’t be provoked/ consoledautonomic changesEEG less predictable
  • 24. Neonatal seizureEtiology– hypoxic-ischemic encephalopathy Presents within first day– congenital CNS anomalies– intracranial hemorrhage– electrolyte abnormalities – hypoglycemia and hypocalcemia– infections– drug withdrawal– pyrodoxine deficiency
  • 25. Status EpilepticusDefinition– Seizure lasting >30 mins– sequential seizures without regain LOC >30minMortality in pediatric status epilepticus 4%Morbidity may be as high as 30%
  • 26. TreatmentCorrect underlying pathology, if anyAntipyretics ineffective in febrile seizureAnti-epileptic choice often trial and error no anti-epileptic 100% effective febrile seizure – diazepam, phenobarbital, valproic acid – Currently AAP does not recommend neonatal - phenobarbital generalized TC – phenytoin, phenobarbital, carbamazepine, valproic acid, primidone absence – ethosuximide, valproic acid new anti-epileptics – felbamate, gabapentin, lamotrigine, topiramate, tiagabine, vigabatrinein consultation with neurologist
  • 27. NeuroimagingNeuroimaging (preferably MRI) is indicated forany child with – epilepsy occurring in the first year of life, except febrile seizures – partial epilepsy except benign rolandic epilepsy – developmental delay or regression
  • 28. EEGEEG is important to support the:– clinical diagnosis of epileptic seizures,– Classify the epileptic syndrome,– selection of anti-epileptic drug and prognosis– helps in localization of seizure foci in intractable epilepsyConsider in:– persistent altered mental status (?non convulsive status epilepticus)– paralyzed patients– pharmacologic coma
  • 29. Dispositioncan be discharged home if– single seizure– stable, returning to baseline neuro status– no underlying condition/cause requiring treatment in hospital– arranged follow-up
  • 30. During development (Congenital, acquired)– Meningitis– Seizure– Headache– Stroke/Vascular– Neoplasm/Tumour– Trauma– Coma
  • 31. HeadachesOccurs in 20% of school-age children. Onset mayoccur at any ageThe most common causes of headache inchildren: - benign vascular headaches (leading to migraine) - muscle contraction (leading to tension headaches)
  • 32. Headaches: classificationClassify based on temporal pattern– acute headaches any febrile illness, sinus/dental infection, intracranial infection/bleed (AVM,SAH,trauma)– acute recurrent– chronic progressive– chronic non-progressive tension, psychogenic, post-traumatic, ocular refractive error
  • 33. Headaches: EtiologiesVascular / Organic Causes- Arteriovenous malformation- Berry aneurysm- Cererbral infarction- Intracranial hemorrhageOther causes– Infection– Trauma– Toxic Effects– Psychogenic– Organic -Traction– Food allergy or sensitivity– Refractive error– Ocular muscle imbalance– Temporomandibular joint (TMJ) dysfunction– Hypertension
  • 34. Acute Recurrent Headachemigraineother– cluster headache – typically >10 yo– sinusitis– vascular malformation
  • 35. Organic –Traction Result of an abnormality in the brain or skull- Headaches increase rapidly in frequency and severity- Headache is worst upon awakening in the morning, diminishes during the day- Headache wakens child from sleep- Aggravated by coughing or valsalva maneuver- May be relieved by vomiting- Associated symptoms: focal neurological findings; altered gait; changes in behavior, personality, cognition or learning ability
  • 36. Migraine- Headache -pulsatile (throbbing)- Headaches are periodic, separated by symptom- free intervals- Associated with at least three of the following symptoms: abdominal pain and nausea or vomiting, aura (motor, sensory, visual), family history of migraine- Unilateral or bilateral- Headache relieved by sleep
  • 37. Tension Headache- Band-like tightness or pressure in the bifrontal, occipital or posterior cervical regions- Seen at any age- Lasting for days or weeks but not disrupting regular activities- Not associated with a prodrome- Associated symptoms: tight neck muscles, sore scalp, nausea, vomiting and aura are uncommon
  • 38. Refractive Error- Persistent frontal headache, which is worse while reading or doing schoolwork
  • 39. TMJ Dysfunction- Temporal headache- Associated symptoms: local jaw discomfort, malocclusion (crossbite), decreased range of motion of mouth, click with jaw movement, bruxism (grinding of teeth)
  • 40. Chronic Sinusitis- Frontal headache- Tenderness to percussion over the frontal, maxillary or nasal sinuses- Associated symptoms: prolonged rhinorrhea and congestion, chronic cough and postnasal drip, anorexia, low-grade fever, malaise- It is unusual for children <10 years old to have recurrent headaches secondary to chronic sinusitis
  • 41. Chronic Progressive Headacheleast common presentationclassically based on historical and physical – sudden severe headache – rapid increase over days - weeks – suggestive of increased ICP severe nocturnal headache (wakes or upon waking), changes in pain with position, coughing – pseudotumor cerebri – space occupying lesion – following head traumaImaging – CT – MRI preferred in non-urgent indication
  • 42. During development (Congenital, acquired)– Meningitis– Seizure– Headache– Stroke/Vascular– Neoplasm/Tumour– Trauma– Coma
  • 43. Stroke/VascularArteriovenous malformationCerebrovascular accident, or stroke
  • 44. Arteriovenous malformationsCerebral arteriovenousmalformations occur in about 1 in100, 000 of the populationThey are the most commoncerebrovascular lesions in children.AVMs are congenital lesions whichoccur as a result of a malfunctionin the normal separation of arteriesand veins during embryonicdevelopment.AVMs in the CNS are more commonabove the tentorium, particularlyaround the middle cerebral artery.
  • 45. Arteriovenous malformationsThe majority of AVMs are asymptomatic.Often these malformations are only discoveredincidentally, usually during treatment for anunrelated disorder.AVMs that are symptomatic usually present inyoung adulthood; the average age ofpresentation in children is about 10 years of age.
  • 46. Arteriovenous malformationsGeneralized symptoms include seizures andheadaches, additionally, children with an AVMsmay present with a range of specific focalneurological signs depending on the location ofthe lesion, such as – muscle weakness or hemiplegia, – loss of coordination, – visual disturbances, – abnormal sensations or – alterations in cognitive functioning.Approximately 50 to 80 per cent of AVMs presentwith signs and symptoms of raised intracranialpressure as a result of intracranial haemorrhage
  • 47. Arteriovenous malformationsTreatment options: – Microsurgery – Endovascular embolization – Radiotherapy
  • 48. Cerebrovascular Accident (stroke)Defined as a clinical syndrome characterized by focalneurological deficits caused by a sudden disruption of theblood supply to the brain, lasting more than 24 hoursStroke is much less common in children than in adults.The estimated incidence of stroke in children isapproximately 3 in every 100,000 childrenStroke in children is more likely to occur in the presence ofunderlying diseases, such as metabolic disorders,haematological disorders (particularly sickle cell anaemia),congenital cardiac disease and moyamoya disease
  • 49. Cerebrovascular Accident (stroke)General care of a child who has had a stroke includesmaintaining normal temperature, ensuring adequateoxygenation and consideration of aspirin in ischaemicstrokeIn the case of moyamoya disease improvements to cerebralblood flow, as a result of intracranial internal carotidocclusion, can be made by performing vasoreconstructivesurgery (bypass surgery).The outcome following stroke in children is variable butsurvival is thought to be better than in adultsThe survival rate following stroke in children has beenestimated to be about 85 per cent
  • 50. During development (Congenital, acquired)– Meningitis– Seizure– Headache– Stroke/Vascular– Neoplasm/Tumour– Trauma– Coma
  • 51. Childhood MalignanciesCancer is the most common cause of diseaserelated deaths in children 1-19 yearsIncidence has increased slowly, but mortalityrates have declined significantly
  • 52. Brain TumoursPrimarily infratentorial involving cerebellum,midbrain, brainstemGlial( cerebellar astrocytomas most common)Presentation - Poor feeding, Vomiting , FTT( failure to thrive) - Arrest or regression of developmental milestones - Morning headache, increased head circumference ( hydrocephalus) - Diplopia, nystagmus, papilloedema - Focal neuro deficits (+Cranial nerves), seizures, ataxia
  • 53. Brain Tumours: Diagnosis and MxComprehensive history and complete PECareful CNS examRule out other causes -infection/trauma/metabolicCT head and/or MRIReferral to neurosurgery
  • 54. During development (Congenital, acquired)– Meningitis– Seizure– Headache– Stroke/Vascular– Neoplasm/Tumour– Trauma– Coma
  • 55. Head InjuriesHISTORY-Ascertain the following:- Mechanism of injury- Time of injury- Loss of consciousness (a brief seizure at the time of injury) may not be clinically significant- Loss of memory , amnesia- Irritability- Visual disturbance- Disorientation- Abnormal gait- Lethargy, pallor or agitation may indicate severe injury- Vomiting- Symptoms of increased intracranial pressure (vomiting, headache, irritability)
  • 56. Head InjuriesPhysical Examination - Vital Signs - Tachypnea: - Bradycardia (with hypertension - Cushing response): - Hypertension - Hypotension
  • 57. Head InjuriesSigns of Skull Fracture - Hemotympanum - Periorbital or post-auricular ecchymosis - Cerebrospinal fluid otorrhea or rhinorrhea - Depressed fracture or penetrating injury - Palpate scalp for hematomas and contusions, underlying depressions, which may signify depressed skull fracture
  • 58. Head InjuriesNeurologic Examination - Pediatric Glasgow coma scale - Papilloedema - Pupillary light reflexes - Cranial nerve examination - Movement of extremities - Abnormal posture (decorticate or decerebrate) - Muscle flaccidity, spasticity - Plantar responses
  • 59. Head InjuriesMild GCS >12Moderate GCS 9-12Severe GCS <9
  • 60. Management Mild InjuryChildren with mild intracranial injury may bedischarged homeAn instruction sheet should be given to theparents or caregiver concerning observation andprecautions
  • 61. Table 15-3: Instructions to Parents or Caregivers forObservation at Home of Children with Head TraumaBring child back to clinic immediately if any of thefollowing signs and symptoms appear within the first72 hours after discharge: Any unusual behavior Disorientation as to name and place Inability to wake child from sleep Increasing headache Seizures Unsteadiness on feet Unusual drowsiness and sleepiness Vomiting more than two or three times
  • 62. Moderate To Severe Injury- ABC’s first priority- C-spine control- Suture scalp lacerations, as major blood loss can occur- Start IV therapy with normal saline to keep vein open (unless the child is in shock from other injuries)- Restrict fluids to 60% of normal intake (except in cases of shock)- Oxygen- Elevate head of bed by 30° to 45°- Place head and neck in midline position
  • 63. Moderate To Severe Injury- Minimize stimuli (e.g., suctioning and movement)- To control increased intracranial pressure: above measures plus establish controlled hyperventilation- CT scan of head- C-spine x-ray- Diuretics if intracranial pressure is increased (and there is documented deterioration) despite measures outlined above:mannitol, 0.5-1 g/kg IV- Monitor ABCs, vital signs, pulse oximetry, level of consciousness (with serial pediatric Glasgow coma scores), intake and output
  • 64. Pediatric Glasgow Coma Scale
  • 65. During development (Congenital, acquired)– Meningitis– Seizure– Headache– Stroke/Vascular– Neoplasm/Tumour– Trauma– Coma
  • 66. Pediatric ComaMost common pattern in children is diffuseimpairment of cerebral hemispheresLess commonly results from brainstemdysfunctionDifferential Dx of causes: – Trauma – Insulin/hypoglycemia/inborn errors of metabolism/intususception – Psychiatric – Seizures, stroke, shock, shunt malfunction – Alcohol abuse – Electrolytes, encephalopathy, endocrinopathy – Infection – Overdose/ingestion – Uremia
  • 67. Pediatric Coma-Initial Approach Primary Survey- ABC;s - C-spine precautions- Pediatric Glasgow Coma Scale- Vital signs including rectal temperature- Check for signs of obvious trauma- Check for S&S of raided ICP- Hypoglycemia- give glucose 0.5 g/kg( D50W, 1-2 ml/kg IV empirically - chemstrip sugar low- Narcan empirically 0.1 mg/kg if pupils small/pinpoint
  • 68. Pediatric Coma-Initial Approach Secondary survey- History - known underlying cause, acute fever, trauma, ingestion, PMH, Medications, allergies, last meal- General CPX including CNS exam- Look for evidence of infection, intoxication, traumatic and metabolic causes- Fontanelle, neck stiffness, neck bruits, fundi( retinal hemorrhages), oculomotor movements- Breathing patterns- Motor responses ( focalizing/lateralizing signs)
  • 69. Pediatric Coma-Initial ApproachInvestigations - depends on potential etiologyand clinical condition– Blood work may include: - CBC, cultures, glucose, electrolytes, BUN, creatinine, Calcium, magnesium, LFT’s, ammonium, blood clotting screen, ABG– Diagnostic Imaging - CT of head essential if focal causes suspected e.g trauma not if diffuse cause e.g infection - CRX, C-spine XR, Flat plate of Abdomen, limb XR - Urinalysis, C&S, latex agglutination - LP- CSF analysis - ECG - EEGFurther management directed at underlying causeSerial Glasgow coma scale assessmentsMaintain homeostasis with Oxygen, IV fluids, electrolytes,nutrition
  • 70. During development (Congenital, acquired)– Meningitis– Seizure– Headache– Stroke/Vascular– Neoplasm/Tumour– Trauma– Coma
  • 71. Thank You